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Crystal Structure of Human Lyase in complex with inhibitor HG-CoA

Source organism: Homo sapiens
Biochemical function: metal ion binding
Biological process: ketone body biosynthetic process
Cellular component: protein-containing complex

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EC 4.1.3.4: Hydroxymethylglutaryl-CoA lyase

Reaction catalysed:
(S)-3-hydroxy-3-methylglutaryl-CoA = acetyl-CoA + acetoacetate
Systematic name:
(S)-3-hydroxy-3-methylglutaryl-CoA acetoacetate-lyase (acetyl-CoA-forming)
Alternative Name(s):
  • (S)-3-hydroxy-3-methylglutaryl-CoA acetoacetate-lyase
  • 3-hydroxy-3-methylglutarate-CoA lyase
  • 3-hydroxy-3-methylglutaryl CoA cleaving enzyme
  • 3-hydroxy-3-methylglutaryl coenzyme A lyase
  • 3-hydroxy-3-methylglutaryl-CoA lyase
  • HMG-CoA lyase
  • Hydroxymethylglutaryl coenzyme A lyase
  • Hydroxymethylglutaryl coenzyme A-cleaving enzyme

GO terms

Sequence family

Pfam Protein family (Pfam)
PF00682
Domain description: HMGL-like
Occurring in:
  1. Hydroxymethylglutaryl-CoA lyase, mitochondrial
The deposited structure of PDB entry 3mp3 contains 6 copies of Pfam domain PF00682 (HMGL-like) in Hydroxymethylglutaryl-CoA lyase, mitochondrial. Showing 1 copy in chain A.

InterPro InterPro annotations
IPR000891
Domain description: Pyruvate carboxyltransferase
Occurring in:
  1. Hydroxymethylglutaryl-CoA lyase, mitochondrial
IPR000138
Domain description: Hydroxymethylglutaryl-CoA lyase, active site
Occurring in:
  1. Hydroxymethylglutaryl-CoA lyase, mitochondrial
IPR043594
Domain description: HMG-CoA lyase
Occurring in:
  1. Hydroxymethylglutaryl-CoA lyase, mitochondrial
IPR013785
Domain description: Aldolase-type TIM barrel
Occurring in:
  1. Hydroxymethylglutaryl-CoA lyase, mitochondrial

Structure domain

CATH CATH domain
3.20.20.70
Class: Alpha Beta
Architecture: Alpha-Beta Barrel
Topology: TIM Barrel
Homology: Aldolase class I
Occurring in:
  1. Hydroxymethylglutaryl-CoA lyase, mitochondrial
The deposited structure of PDB entry 3mp3 contains 6 copies of CATH domain 3.20.20.70 (TIM Barrel) in Hydroxymethylglutaryl-CoA lyase, mitochondrial. Showing 1 copy in chain A.

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